Brief Article
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World J Gastroenterol. Feb 21, 2013; 19(7): 1111-1118
Published online Feb 21, 2013. doi: 10.3748/wjg.v19.i7.1111
Risk factors for hepatic decompensation in patients with primary biliary cirrhosis
Tian-Yan Shi, Li-Na Zhang, Hua Chen, Li Wang, Min Shen, Xuan Zhang, Feng-Chun Zhang
Tian-Yan Shi, Li-Na Zhang, Hua Chen, Li Wang, Min Shen, Xuan Zhang, Feng-Chun Zhang, Department of Rheumatology, Key Laboratory of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing 100032, China
Author contributions: Zhang FC designed the research plan; Chen H, Wang L, Shen M, Zhang X, and Zhang FC recruited patients; Shi TY and Zhang LN analyzed data; Shi TY wrote the paper.
Supported by National Science Technology Pillar Program in the 11th Five-Year Plan, No. 2008BAI59B03; and Research Special Fund for the Public Welfare Industry of Health, No. 201202004
Correspondence to: Feng-Chun Zhang, MD, Department of Rheumatology, Key Laboratory of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No. 41 Da Mu Cang, Western District, Beijing 100032, China. zhangsamfch@hotmail.com
Telephone: +86-10-88068795 Fax: +86-10-88068794
Received: October 29, 2012
Revised: January 2, 2013
Accepted: January 11, 2013
Published online: February 21, 2013
Processing time: 115 Days and 23.6 Hours
Abstract

AIM: To examine the clinical features and analyze prognostic factors in a prospective study of primary biliary cirrhosis (PBC) patients.

METHODS: From 1995 to 2010, PBC patients without hepatic decompensation seen at the Peking Union Medical College Hospital were enrolled. Clinical signs and manifestations (pruritus, persistent fatigue, jaundice and pain in the right hypochondrium), laboratory parameters (auto-antibodies for autoimmune hepatic disease, biliary and hepatic enzymes, immunoglobulin, bilirubin, and albumin) and imaging findings were recorded at entry and at specific time points during follow-up. Cox regression and Kaplan-Meier analyses, respectively, assessed the risk factors for hepatic decompensation and survival.

RESULTS: Two hundred and sixty-two PBC patients were enrolled with a median follow-up of 75.2 mo (range, 21-201 mo). The 240 patients were aged 51.5 ± 10.2 years at diagnosis and 91.6% were female. Two hundred and forty-five (93.5%) were seropositive for anti-mitochondrial antibodies. At presentation, 170 patients (64.9%) were symptomatic, while 96 patients (36.6%) had extra-hepatic autoimmune disease. During the follow-up period, 62 (23.7%) patients developed hepatic decompensation of whom four underwent liver transplantation and 17 died. The cumulative survival rate and median survival time were 83.9% and 181.7 mo, respectively. Cox regression analysis revealed that an incomplete ursodeoxycholic acid (UDCA) response or inconsistent treatment [P < 0.001; hazard risk (HR) 95%CI = 2.423-7.541], anti-centromere antibodies (ACA) positivity (P < 0.001; HR 95%CI = 2.516-7.137), alanine aminotransferase ratio (AAR) elevations (P < 0.001; HR 95%CI = 1.357-2.678), and histological advanced liver disease (P = 0.006; HR 95%CI = 1.481-10.847) were predictors of hepatic decompensation. The clinical features and survival of PBC in China are consistent with those described in Western countries.

CONCLUSION: Incomplete UDCA response or inconsistent treatment, ACA positivity, AAR elevations, and advanced histological stage are predictors of decompensation.

Keywords: Primary biliary cirrhosis; Risk factor; Hepatic decompensation; Survival; Ursodeoxycholic acid response; Anti-centromere antibodies; Histological stage